This project will test the hypothesis that carnitine biosynthesis occurs by conversion 6-N-Trimethyllysine to 4-N-Trimethylaminobutyrate which is then hydroxylated to carnitine. In vivo experiments show that glycine is a primary fission product which implicated 3-hydroxytrimethyllysine, the first product of trimethyllysine. The objective is to characterize the products and enzymes involved in hydroxylation, aldolase cleavage and gamma-butyrbetaine aldehyde dehydrogenation, the three steps not understood in the synthesis of carnitine from lysine.